Trend over time

In 2010, there were 3,272 new cases of STS (all subtypes combined) in the UK: 1,660 (51%) in males and 1,612 (49%) in females.[1] The crude incidence rate shows that there are 54 new STS cases for every million males in the UK, and 51 for every million females.[1] Males in the UK have a similar risk of developing STS compared with females; the male:female incidence ratio of European age-standardised rates (AS rates), which account for differing male and female age distribution and population sizes, is 11:10.

The European age-standardised incidence rates (AS rates) do not differ significantly between the constituent countries of the UK.[1]

Soft Tissue Sarcoma (All Subtypes Combined), Number of New Cases, Crude and European Age-Standardised (AS) Incidence Rates per Million Population, UK, 2010

95% LCL and 95% UCL are the 95% lower and upper confidence limits around the AS rate

A fifth of all STS cases diagnosed in the UK are sarcoma not otherwise specified (NOS, 20%) – cases where the pathologist reporting the case, or the cancer registry recording the tumour, did not enter a specific morphological soft tissue sarcoma sub-type.[1] Almost a fifth of cases (18%) are leiomyosarcoma, while fibroblastic sarcoma and liposarcoma account for 14% and 13% respectively. The remaining 34% of STS cases is comprised of eight other morphological groups, including one group which is itself comprised of 42 morphologies which cannot be assigned to a larger sub-group (rare soft tissue sarcoma variants). Crude incidence rates range from 0.0 (malignant phyllodes tumour – tumours of breast tissue which are extremely rare in men) to 11 (sarcoma NOS) cases for every million males in the UK, and from 1 (soft tissue Ewing sarcoma, nerve sheath tumour, Kaposi sarcoma, synovial sarcoma) to 11 (leiomyosarcoma) cases for every million females.[1]

European AS incidence rates are significantly higher in males than females for five of the 12 STS subtypes (liposarcoma, fibroblastic sarcoma, rhabdomyosarcoma, Kaposi sarcoma, sarcoma NOS). In a further five subtypes (soft tissue Ewing sarcoma, synovial sarcoma, vascular sarcoma, nerve sheath tumours, rare soft tissue sarcoma variants) rates do not differ significantly between the sexes. Rates are significantly higher in females than males for leiomyosarcoma and malignant phyllodes tumour only.[1]

Soft Tissue Sarcoma Subtypes, Number of New Cases and European Age-Standardised (AS) Incidence Rates per Million Population, UK, 2008-2010

Male

Female

Persons

Cases

AS Rate (95% LCL- 95% UCL)

Cases

AS Rate (95% LCL- 95% UCL)

Cases

AS Rate (95% LCL- 95% UCL)

Leiomyosarcoma

248

6.8 (6.3 - 7.3)

355

9.5 (8.9 - 10.1)

603

8.1 (7.8 - 8.5)

Liposarcoma

275

7.9 (7.4 - 8.5)

163

4.4 (4.0 - 4.8)

438

6.2 (5.8 - 6.5)

Fibroblastic sarcoma

245

7.1 (6.6 - 7.6)

214

5.9 (5.4 - 6.4)

459

6.5 (6.1 - 6.8)

Rhabdomyosarcoma

72

2.5 (2.2 - 2.9)

49

1.7 (1.4 - 2.0)

121

2.1 (1.9 - 2.3)

Soft tissue Ewing sarcoma

19

0.7 (0.5 - 0.9)

21

0.7 (0.5 - 0.9)

40

0.7 (0.6 - 0.8)

Synovial sarcoma

39

1.3 (1.0 - 1.5)

42

1.3 (1.1 - 1.6)

81

1.3 (1.1 - 1.5)

Vascular sarcoma

60

1.6 (1.3 - 1.8)

86

2.0 (1.8 - 2.3)

146

1.8 (1.6 - 2.0)

Karposi sarcoma

125

3.9 (3.6 - 4.4)

27

0.8 (0.6 - 1.0)

152

2.4 (2.2 - 2.6)

Nerve sheath tumour

43

1.3 (1.1 - 1.6)

36

1.0 (0.9 - 1.3)

80

1.2 (1.0 - 1.3)

Malignant phyllodes tumour

0

0.0 (0.0 - 0.1)

62

1.7 (1.5 - 2.0)

62

0.9 (0.7 - 1.0)

Rare soft tissue sarcoma variants

189

5.4 (5.0 - 5.9)

232

6.1 (5.6 - 6.6)

421

5.8 (5.4 - 6.1)

Sarcoma NOS

339

9.2 (8.6 - 9.8)

322

7.7 (7.2 - 8.2)

661

8.5 (8.1 - 8.9)

95% LCL and 95% UCL are the 95% lower and upper confidence limits around the AS rate

References

Soft tissue sarcoma (STS) (all subtypes combined) incidence is unusual compared with most cancers because a sizeable proportion of cases occur in children and younger adults; however the highest incidence rates are in older men and women. In the UK between 2008 and 2010, an average of 43% of cases were diagnosed in men and women aged 65 years and over, but almost 1 in 10 (9%) were diagnosed in the under-30s.[1]

Age-specific incidence rates rise steadily from childhood, increasing more rapidly from around age 55-59 in men, but continuing at a similar pace throughout in women. Rates are slightly higher in 0-4 year-olds than in older children, due to the incidence of rhabdomyosarcoma in this age group. Incidence rates are similar for males and females in most age groups, though in the oldest age groups (75-79 and older) male rates are significantly higher than female rates. Female rates overtake male rates (though the difference is not significant) for a short period of early middle age, due to the incidence of gynaecological sarcoma at this time.[1]

Soft Tissue Sarcoma (All Subtypes Combined), Average Number of New Cases per Year and Age-Specific Incidence Rates, UK, 2008-2010

The age profile of soft tissue sarcoma (STS) cases in the UK varies markedly by subtype. For example, people aged 60 years and over comprise around 74% of vascular sarcoma cases, but only around 43% of malignant phyllodes tumour cases.[1] Around two thirds (67%) of sarcoma not otherwise specified (NOS) cases are in people aged 60 and over, probably because older people are less likely to have surgery, meaning pathological samples required to identify the specific morphology sub-type are not collected.[2]

Age-specific incidence rates increase with age in most soft tissue sarcoma (STS) subtypes, with the steepest rise for vascular sarcoma (males and females), and slowest rises for liposarcoma (males) and fibroblastic sarcoma (females). Rates peak in the 80+ age group for most subtypes, but peak in the 60-69 age group for liposarcoma and malignant phyllodes tumours in females (the latter probably because this age group is routinely invited for breast screening in the UK), and in the 70-79 age group for leiomyosarcoma and rare soft tissue variants in females, and liposarcoma in males.[1]

Incidence rates are similar between males and females in most age groups for most subtypes, though the sex ratios vary. In the few age groups and subtypes with significant sex differences, rates are typically higher in males than females; the exception is leiomyosarcoma, where rates are higher in females than males in the 0-49 and 50-59 age groups.[1]

Improved diagnostic techniques and more accurate recording within the cancer registries has impacted on soft tissue sarcoma (STS) incidence rates over time.[1] Because of this, long-term trends are not reliable, so in this section trends are presented from 1996 onwards. Trends vary markedly by STS subtype, perhaps in part because the impact of diagnosis and coding changes also varies with subtype.

STS (all subtypes combined) incidence rates have increased overall in the UK since the mid-1990s.[2] For males, European age-standardised (AS) incidence rates increased by 18% between 1996-1998 and 2008-2010. The rise is slightly smaller for females, with rates increasing by 14% between 1996-1998 and 2008-2010. Over the last decade (between 1999-2001 and 2008-2010), incidence rates have increased by 17% in males, but have not changed significantly in females.

Soft tissue sarcoma (STS) (all subtypes combined) incidence rates have increased overall for some of the broad age groups in the UK since the mid-1990s, and have remained stable for others.[2] In people aged 49 and under, and those aged 60-69, European AS incidence rates increased by 18% and 22%, respectively, between 1996-1998 and 2008-2010. In the other broad age groups, rates did not change significantly.

Incidence rates have remained stable for most of the Soft tissue sarcoma (STS) subtypes in the UK since the mid-1990s.[2]

For rhabdomyosarcoma, soft tissue Ewing sarcoma, nerve sheath tumour, and malignant phyllodes tumour, European AS incidence rates remained stable between 1996-1998 and 2008-2010.[2]

For liposarcoma, fibroblastic sarcoma, rare soft tissue sarcoma variants, and sarcoma not otherwise specified (NOS), European age standardised (AS) incidence rates increased significantly between 1996-1998 and 2008-2010. For synovial sarcoma and Kaposi sarcoma, rates increased between 1996-1998 and 2003-05 and 1996-1998 and 2006-2008 respectively, but have since remained stable. For vascular sarcoma, the absolute increase in rates was not quite statistically significant.

The size of the increase ranges from 21% (sarcoma NOS) to 103% (rare soft tissue sarcoma variants).[2] In some STS subtypes this increase reflects expansion of the subtype definition; for example once previously non-registerable tumours called atypical lipomatous tumours were included with well-differentiated liposarcoma, liposarcoma incidence rates began to rise.[1]

Conversely, for leiomyosarcoma there has been a significant 24% decrease between 1996-1998 and 2008-2010. This probably reflects narrowing of the subtype definition; in the year 2000 gastro-intestinal stromal tumours (GISTs) were classified separately from leiomyosarcoma in ICD-O-3, and leiomyosarcoma incidence rates began to fall.[1]

Within some STS subtypes, for example liposarcoma and fibroblastic sarcoma, incidence rates of the ‘not otherwise specified’ (NOS) subtype have decreased as rates of other specific subtypes have increased, probably reflecting changes in reporting practice within pathology laboratories which allow more specific coding by cancer registries. There has also been some migration of cases across STS subtypes, for example Ewing sarcoma incidence rates increased during the early- and mid-1990s when the chromosomal translocations characteristics of this subtype were identified. Prior to this; Ewing sarcoma may have been diagnosed as another round cell sarcoma type.[1]

However, increasing incidence of some STS subtypes is probably attributable to changing aetiological factors; for example angiosarcoma (a type of vascular sarcoma) of the breast is increasingly common (though still exceptionally rare), perhaps due to the growing number of women undergoing radiotherapy for breast cancer,[3] and Kaposi sarcoma incidence rates have fluctuated in line with HIV infection rates.[1]

The largest proportion of soft tissue sarcoma cases occur in the limbs, with slightly smaller proportions in the connective tissue of the trunk, and a much smaller proportion in the gynaecological organs (2008-2010).[1]

A moderate proportion of cases did not have the specific part of the sarcoma recorded.[1]

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